Abstract
Primary erythromelalgia (PE) is a refractory pain disorder characterized by the triads of pain, redness, and heat in the extremities induced by local exposure to warmth. Here we report a successful treatment for a 54-year-old woman who suffered from triads of PE for ten years. Originally, she underwent intermittent severe pain attacks twenty times per day and had sleep disorder. The symptoms showed remarkable improvements by stellate ganglion block of 14 times. Subsequently, sustaining oral administration of alpha-1 adrenergic antagonist keeps the symptoms in stable condition. Previous studies showed that PE patients have mutations in the gene encoding the sodium channel Nav1.7 expressed within the dorsal root ganglion and sympathetic ganglion neurons. These observations suggested that this increases the excitability in neurons and causes low pain thresholds and peripheral ischemia. Meanwhile this generates neuropeptide and chemical mediator releases and causes peripheral redness. Consistent with prior reports, our experience suggests that PE has excessive activities in a sympathetic nervous system.
